Pediatric Polycythemia Vera Medication

  • Author: Josef T Prchal, MD; Chief Editor: Robert J Arceci, MD, PhD   more...
 
Updated: Apr 13, 2012
 

Medication Summary

Chemotherapeutic cytoreductive therapy is used in all patients who are high risk but not in those who are low risk. All patients receive low dose aspirin.

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Biologic response modifiers

Class Summary

Biologic response modifiers elicit antiproliferative, antiviral, and immunomodulating effects. They inhibit cellular growth and alter cellular differentiation.

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Interferon alfa-2a, recombinant (Pegasys)

 

Protein produced in response to viral infection and other inflammatory stimuli. The exact mechanism is unknown, but it is believed to exert an antiproliferative effect. Produced by recombinant DNA techniques in E coli. Controls erythrocytosis and reduces spleen size. Unlike hydroxyurea or anagrelide, can also ameliorate the intractable pruritus often found in polycythemia vera. Sporadic case reports have noted cytogenetic remissions, suggesting a possible biologic effect.

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Antiplatelet agents

Class Summary

These agents prevent formation of thrombi-associated polycythemia.

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Aspirin (Anacin, Ascriptin, Bayer)

 

Irreversibly acetylates platelet cyclooxygenase, resulting in a decrease in thromboxane A2, the prostaglandin responsible for platelet shape change, granule release, and aggregation. Prescribed for most patients with polycythemia vera.

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Anagrelide (Agrylin)

 

Inhibits post mitotic megakaryocyte maturation. Unlike hydroxyurea, selectively inhibits platelet proliferation. In polycythemia vera used only to control platelet counts, but shows slight decrease in mean hemoglobin and hematocrit while white cell counts maintained. Inhibits cyclic nucleotide phosphodiesterase and the release of arachidonic acid from phospholipase, possibly by inhibiting phospholipase A2. Can be used in addition to hydroxyurea for particularly difficult to control thrombocytosis in polycythemia vera.

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Antineoplastic Agent

Class Summary

These agents are used off-label for polycythemia, but pediatric doses are extrapolated from pediatric treatment regimens, including leukemia and myelodysplastic syndrome.

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Hydroxyurea (Droxia, Hydrea)

 

Nonalkylating, myelosuppressive, S-phase agent. Inhibits ribonucleotide reductase, the enzyme that converts ribonucleotides into deoxynucleotides, thereby depleting deoxynucleotide and inhibiting DNA synthesis. Cellular proliferation is ultimately inhibited, and leukocytes, erythrocytes, and platelets are decreased. The mechanism of action is probably different than the one exerted by hydroxyurea in the treatment of sickle cell disease. A misconception is that hydroxyurea is leukemogenic. No studies have conclusively demonstrated that hydroxyurea is more leukemogenic than baseline in myeloproliferative disease.

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Contributor Information and Disclosures
Author

Josef T Prchal, MD  Professor, Department of Medicine, Division of Hematology, Adjunct Professor of Pathology and Genetics, University of Utah School of Medicine

Josef T Prchal, MD, is a member of the following medical societies: American College of Physicians, American Society of Hematology, American Society of Human Genetics, Association of American Physicians, and Southern Society for Clinical Investigation

Disclosure: Nothing to disclose.

Coauthor(s)

Scott J Samuelson, MD  Fellow in Hematology and Oncology, Huntsman Cancer Institute, University of Utah School of Medicine

Scott J Samuelson, MD is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine and American Society of Hematology

Disclosure: Nothing to disclose.

Specialty Editor Board

Sharada A Sarnaik, MBBS  Professor of Pediatrics, Wayne State University School of Medicine; Director, Sickle Cell Center, Attending Hematologist/Oncologist, Children's Hospital of Michigan

Sharada A Sarnaik, MBBS is a member of the following medical societies: American Association of Blood Banks, American Association of University Professors, American Society of Hematology, American Society of Pediatric Hematology/Oncology, New York Academy of Sciences, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

James L Harper, MD  Associate Professor, Department of Pediatrics, Division of Hematology/Oncology and Bone Marrow Transplantation, Associate Chairman for Education, Department of Pediatrics, University of Nebraska Medical Center; Assistant Clinical Professor, Department of Pediatrics, Creighton University School of Medicine; Director, Continuing Medical Education, Children's Memorial Hospital; Pediatric Director, Nebraska Regional Hemophilia Treatment Center

James L Harper, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Federation for Clinical Research, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Council on Medical Student Education in Pediatrics, and Hemophilia and Thrombosis Research Society

Disclosure: Nothing to disclose.

Helen SI Chan, MBBS, FRCP(C), FAAP  Associate Senior Scientist, Research Institute; Professor, Division of Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto Faculty of Medicine, Canada

Helen SI Chan, MBBS, FRCP(C), FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Society of Hematology, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Chief Editor

Robert J Arceci, MD, PhD  King Fahd Professor of Pediatric Oncology, Professor of Pediatrics, Oncology and the Cellular and Molecular Medicine Graduate Program, Kimmel Comprehensive Cancer Center at Johns Hopkins University School of Medicine

Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, and American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

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Bone marrow film at 400X magnification demonstrating dominance of erythropoiesis. Courtesy of U. Woermann, MD, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland.
 
 
 
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